Neuronal ceroid lipofuscinosis: clinical and morphologic findings in nine affected Polish Owczarek Nizinny (PON) dogs     

Narfström K  Wrigstad A  Ekesten B  Berg AL 《Veterinary ophthalmology》2007,10(2):111-120

OBJECTIVE: The aim of this study was to characterize the clinical and morphologic features of neuronal ceroid lipofuscinosis (NCL) in the Polish Owczarek Nizinny (PON) breed of dog. ANIMALS: Nine Swedish PON dogs of both sexes were included in the study. PROCEDURE: All dogs underwent a detailed clinical evaluation, with emphasis on ophthalmic exams. Histopathology and electron microscopy were performed on the eyes, brain and various internal organs. Immunohistochemical staining for detection of sphingolipid activator proteins (SAPs) and mitochondrial ATP synthase (SCMAS) was performed on the eyes and brain. RESULTS: The dogs showed behavioral abnormalities, motor disturbances and visual impairment or blindness. Pupillary responses were abnormal while fundus changes varied from normal to severe retinal atrophy. Electroretinography (ERG) showed variable changes, from slight alterations in the process of dark adaptation to severely reduced or nonrecordable ERG a- and b-wave amplitudes. Histopathology revealed intracytoplasmic storage bodies within neurons of the brain and in retinal cells, especially the retinal pigment epithelium (RPE). Round to oval granular type of inclusion bodies, known as granular osmiophilic dense deposits (GRODS), were found in neuronal cells in the brain and in the retina. Immunohistochemistry identified the storage material in the brain and retina as consisting of SAPs. CONCLUSION: The presently described NCL disease in PON dogs shows similarities to previously recorded cases in the Miniature Schnauzer. The closest human equivalent to this disease is infantile NCL (CLN1), in which the major stored proteins are SAPs and the ultrastructure of the inclusion bodies of neuronal cells is granular.  相似文献   

Nanomaterized zinc sulfide-meerschaum biomatrix efficiently suppressed Fusarium verticilloides with augmented rice seed quality benefits during storage     

Varinder Khepar  Anjali Sidhu  Anju Bala Sharma 《Pest management science》2023,79(1):244-256

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Experimental Investigation on Chip-break Influenced by Cryogenical Cold Air jet     

YANG Ying  TONG Ming-wei  YAN Xing-chun  SHEN Qiang 《保鲜与加工》2004,(5):74-77

In machine cutting, the replacement of traditionalKGcutting solution with cryogenical cold air jet not only possesses a good cooling and lubricating efficiency, but also can avoid the occurrenceof environmental pollution. In order to clarify the manner and performance of discharged cutting chip under cryogenical cold air jet condition, an experiment was carried out for steel grade 45 in the dry and cryogenical cold air jet cutting comparatively. The effects of cryogenical cold air jet on the breaking of cutting chip are discussed. Experimental results reveal that, in the valid ranges of pressure and temperature, the cryogenical cold air jet can widen the chip broken region effectively. Different insufflating angle of cold air behaves different influence on the chip-break. From the experiment, it has been gained that the optimized jet insufflatingangle, temperature effecting range and pressure working range of cold air, which provides the foundation of industrial manufacturing.  相似文献   

Research on Preparation and Hydrogen Storage Properties of Mg2Ni Hydrogen Storage Alloys     

CHEN Yu-an  MIAO He  XU Xin-zi 《保鲜与加工》2005,(12):39-41

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PREDICTIVE INTELLIGENT CONTROL OF THE COLD STORAGE     

Cao Changxiu Wang WanLiang 《保鲜与加工》1989,(5):68-75

The predictive intelligent control scheme is proposed, which is based on approximation theory and numerical method of Chcbyshev orthogonal polynomial. It has the practical value to the cold storge using widely electromagnetic valve and can be also applied similar systems in orther area.  相似文献   

Land Storage System: Effect, Problem and Its Legal Regulation     

HUANG Xi-sheng~  TANG Shao-jun~  WANG Bao-xin~ 《保鲜与加工》2004,(2)

The authors suggest that the land storage system is beneficial not only to strengthen the country's macroscopic regulation of city land market, accelerate healthy development of city economy and increase the financial income, it can also be advantageous to carry out the economic reform and public facilities construction without a hitch. On the basis of this, in this paper, the increment of the land price and house price as well as the high risk of fund due to lack of related laws is analyzed. Corresponding legal regulations, such as polishing the legislation of land right, implementing the land utilization planning, reinforcing the legislation of city development, land requisition and countryside residential land administration are put forward.  相似文献   

A STUDY ON HEAT AND MOISTURE STATE OF HULL LAYER FOR THERMAL INSTULATION OF A COLD STORAGE     

Chen Qigao Feng Ya 《保鲜与加工》1993,(1)

This paper analyses the thermal moist characteristics of the hull layer beside an air layer at lower temperature in an envelope for a cold storage and presents a new method of waterproof by air layer. With test data from engineering practice, the theory on the barrier of self moisture proof at the surface sublayer of the hull layer in an attic on a cold storage is proven. The dried zone about three phase point is confirmed. It is a new knowledge for thermophysics on thermal design of a cold storage with an optimum thermal performance.  相似文献   

以粮温实测数据为例探讨低温储粮的可行性   总被引：1，自引：0，他引：1  

张前 《粮食储藏》2004,33(3):3-6

通过对高大平房仓粮温进行跟踪检测，分析比较了粮温变化情况，并探讨了低温储粮的可行性。  相似文献   

工业循环冷却水降温系统设计     

陈海林  崔志坤  江海斌  张国芳 《农业装备技术》2007,33(4):20-21

在许多工业生产过程中,常需要大量的冷却水进行冷却。通常采用冷却塔等敞开式循环水降温设施,在高温、高湿大气条件下出口水温超限,此时为了正常生产的进行,只好加大低温新水补充量,从而造成水资源的浪费。为了合理利用和节约水资源,设计土壤蓄冷式降温系统。在夜间冷却塔有富余冷量的时候,把冷量储存在土壤中;在白天释放出来,满足高负荷需要。  相似文献   

温室地下贮热系统设计研究   总被引：4，自引：1，他引：4  

王永维  苗香雯  崔绍荣 《农机化研究》2004,(1):137-139

为了降低温室环境调控能耗。根据育苗不同阶段对气温与土壤温度的要求，设计了温室地下贮热系统，并阐述了系统的组成和贮热原理；同时，分析了各设计参数对系统贮热性能的影响。这样不仅为系统设计与理论分析奠定了基础，对其它类似地下贮热系统的设计、研究也具有参考意义。  相似文献